Management of Syndromic Craniosynostosis in Children

Initial Management at Presentation

Evaluate for need for immediate intervention: Each case must be evaluated individually, with the first aim being to prevent elevated ICP, visual compromise, and airway impairment and the second aim being to improve appearance.

Plan for cosmetic reconstruction: If nothing needs to be done acutely, then an elective surgical plan can be made. Consideration is given to the need for skull vault expansion to reduce ICP, fronto-orbital advancement to enlarge orbits, and the like.

Establish follow-up plan: If no elective plan for surgical intervention is made initially, a plan is established for how the patient will be monitored and what changes will lead to a reevaluation of the management plan.

Adjunctive Therapies

Follow-up

Team follow-up into adulthood: Regular follow-up is required from diagnosis to early adulthood. Clinical review, especially plastic surgical, neurosurgical, and ophthalmological review, is required. Annual follow-up is carried out of patients in stable phases of their treatment. In the early phases of treatment or in a period of instability, more frequent review may be necessary.

Radiographic evaluation and ICP recording: In the event of features of concern, particularly related to possible elevated ICP or visual compromise, re-imaging with CT and/or MRI scan and, if necessary, ICP monitoring are carried out.